Zusammenfassung
Die idiopathische Lungenfibrose ist eine chronisch progrediente fibrosierende interstitielle Pneumonie unbekannter Ursache, die primär beim älteren Erwachsenen auftritt und sich radiologisch sowie histologisch durch das Vorliegen eines UIP-Musters (usual interstitial pneumonia) auszeichnet. Die Prognose ist ungünstig und mit der verschiedener onkologischer Erkrankungen vergleichbar. Interindividuell ist der Verlauf jedoch sehr variabel. Die zentrale Säule der Diagnostik ist die hochauflösende Computertomografie, eine Histologiegewinnung ist nur selten erforderlich. Die Diagnosestellung sollte in einer interdisziplinären Fallevaluation erfolgen. Therapeutisch stellt die Lungentransplantation die einzige kurative Option dar. Mit den antifibrotisch wirksamen Substanzen Pirfenidon oder Nintedanib kann eine Progressionsverzögerung erreicht werden. Supportive Ansätze sollten früh in die Behandlung integriert werden.
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Hagmeyer, L., Kolb, M. (2016). Idiopathische Lungenfibrose. In: Kreuter, M., Costabel, U., Herth, F., Kirsten, D. (eds) Seltene Lungenerkrankungen. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48419-7_11
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